[CIS PIDD] [cis-pidd] Fwd: Hyper IgE syndrome

[dmvascon at usp.br]

Dear Luciana, good evening

I think that your description is very suggestive of an AD hyper IgE due to STAT3 mutation.
Can you perform STAT3 phosphorylation by flow? 
It is interesting to look for IL17 production, that is probably very low and at least partially responsible for the susceptibility to Staph infection.
I will look for someone here in our lab who should perform STAT3 phosphorylation and, if positive I contact you.

All the best,

Dewton Vasconcelos
University of São Paulo School of Medicine

----- luciana Cunha <lucianaaoc at gmail.com> escreveu:

> Dear friends:

> 

> I would like to discuss a case.

> We admitted at ICU a 7 year-old girl who was being treated as a severe

> atopic dermatitis, presenting with a staphylococcal and pseudomonas sepsis.

> She also has eosinophilia (>2000/mL), normal immunoglobulins levels, normal

> lymphocyte immunophenotyping ( CD3,CD4, CD8 CD19, CD16/56), primary teeth

> retention, chronic eczema, characteristic face and high palate. She doesn't

> present pneumonias or parenchimal lung anomalies. She is from a

> consanguinous family with many cases of less severe atopic dermatitis.

> At the moment, she is using vancomycin, cefepime, prednisone 1mg/kg/day and

> diphenydramine. We are considering the use of gammaglobulin or omalyzumab.

> What's your opinion? Any other suggestion?

> Thank you very much,

> 

> Luciana Cunha

> Primary Immunodeficiency Service

> Federal University of Minas Gerais

> Brazil

> 

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